Results 111 to 120 of about 6,546,213 (228)

Huntingtin is a cytoplasmic protein associated with vesicles in human and rat brain neurons

, 1995
The gene defective in Huntington's disease encodes a protein, huntingtin, with unknown function. Antisera generated against three separate regions of huntingtin identified a single high molecular weight protein of ∼320 kDa on immunoblots of human ...
Meloni, Alison, Vonsattel, Jean-Paul, Boyce, Frederick M, Sapp, Ellen, Martin, Eileen, Carraway, Robert, Schwarz, Cordula, Reeves, Steven A, Chase, Kathryn, Aronin, Neil, Young, Christine, DiFiglia, Marian   +11 more
core   +1 more source

UBL3 Interacts with PolyQ-Expanded Huntingtin Fragments and Modifies Their Intracellular Sorting

Neurology International
Background/Objectives: UBL3 (Ubiquitin-like 3) is a protein that plays a crucial role in post-translational modifications, particularly in regulating protein transport within small extracellular vesicles. While previous research has predominantly focused
Soho Oyama, Hengsen Zhang, Rafia Ferdous, Yuna Tomochika, Bin Chen, Shuyun Jiang, Md. Shoriful Islam, Md. Mahmudul Hasan, Qing Zhai, A. S. M. Waliullah, Yashuang Ping, Jing Yan, Mst. Afsana Mimi, Chi Zhang, Shuhei Aramaki, Yusuke Takanashi, Tomoaki Kahyo, Yoshio Hashizume, Daita Kaneda, Mitsutoshi Setou   +19 more
doaj   +1 more source

Mutant Huntingtin Forms in Vivo Complexes with Distinct Context-Dependent Conformations of the Polyglutamine Segment

Neurobiology of Disease, 1999
Huntington's disease (HD) is caused by an expanded glutamine tract, which confers a novel aggregation-promoting property on the 350-kDa huntingtin protein.
Francesca Persichetti, Flavia Trettel, C.Chris Huang, Cornel Fraefel, H.T.Marc Timmers, James F. Gusella, Marcy E. MacDonald   +6 more
doaj   +1 more source

Expanded-Polyglutamine Huntingtin Protein Suppresses the Secretion and Production of a Chemokine (CCL5/RANTES) by Astrocytes

Journal of Neuroscience, 2008
Huntington's disease (HD) is a hereditary neurological disease caused by expended CAG repeats in the HD gene, which codes for a protein called Huntingtin (Htt).
Szu-Yi Chou, J. Weng, H. Lai, Fang Liao, Synthia H. Sun, P. Tu, D. Dickson, Y. Chern   +7 more
semanticscholar   +1 more source

Reduced Levels of Proteasome Products in a Mouse Striatal Cell Model of Huntington's Disease.

PLoS ONE, 2015
Huntington's disease is the result of a long polyglutamine tract in the gene encoding huntingtin protein, which in turn causes a large number of cellular changes and ultimately results in neurodegeneration of striatal neurons. Although many theories have
Sayani Dasgupta, Michael A Fishman, Hana Mahallati, Leandro M Castro, Alexandre K Tashima, Emer S Ferro, Lloyd D Fricker   +6 more
doaj   +1 more source

FIP-2, a coiled-coil protein, links Huntingtin to Rab8 and modulates cellular morphogenesis

, 2000
Huntington's disease is characterised by the death of cortical and striatal neurons, and is the result of an expanded polyglutamine tract in the Huntingtin protein [1].
Hattula, Katarina, Katarina Hattula, Peränen, Johan, Johan Peränen   +3 more
core   +1 more source

Proteostasis in striatal cells and selective neurodegeneration in Huntington’s disease

Frontiers in Cellular Neuroscience, 2014
Selective neuronal loss is a hallmark of neurodegenerative diseases, including Huntington’s disease (HD). Although mutant huntingtin, the protein responsible for Huntington’s disease, is expressed ubiquitously, a subpopulation of neurons in the striatum ...
Julia eMargulis, Julia eMargulis, Steven eFinkbeiner, Steven eFinkbeiner, Steven eFinkbeiner   +4 more
doaj   +1 more source

Huntingtin: an iron-regulated protein essential for normal nuclear and perinuclear organelles

, 2000
Huntington’s disease (HD), with its selective neuronal cell loss, is caused by an elongated glutamine tract in the huntingtin protein. To discover the pathways that are candidates for the protein’s normal and/or abnormal function, we surveyed 19 classes ...
PASSANI L. A, AUERBACH A, PERSICHETTI F. AND MACDONALD M. E., TRETTEL, Flavia, HILDTICH MAGUIRE P   +4 more
core   +2 more sources

Detection of alpha-rod protein repeats using a neural network and application to huntingtin.

PLoS Computational Biology, 2009
A growing number of solved protein structures display an elongated structural domain, denoted here as alpha-rod, composed of stacked pairs of anti-parallel alpha-helices.
Gareth A Palidwor, Sergey Shcherbinin, Matthew R Huska, Tamas Rasko, Ulrich Stelzl, Anup Arumughan, Raphaele Foulle, Pablo Porras, Luis Sanchez-Pulido, Erich E Wanker, Miguel A Andrade-Navarro   +10 more
doaj   +1 more source

Calmodulin regulates transglutaminase 2 cross-linking of Huntingtin [PDF]

, 2004
This is the publisher's version, also available electronically from "www.jneurosci.org".Striatal and cortical intranuclear inclusions and cytoplasmic aggregates of mutant huntingtin are prominent neuropathological hallmarks of Huntington's disease (HD ...
Ross, Christopher A., Troncoso, Juan C., Fitzgerald, John K., Zainelli, Gina M., Muma, Nancy A.   +4 more
core   +1 more source